Omar S, Feki M, Kaabachi N
Laboratoire de Biochimie, Hôpital La Rabta, Tunis.
Ann Biol Clin (Paris). 2006 Nov-Dec;64(6):523-34.
The paper is an up to date overview of knowledge on iron metabolism that integrate recent findings in this field. Significant advances were made in understanding the implication of protein factors (transporters, enzymes and regulation factors) in iron metabolism, as well as related genetic abnormalities. The research highlighted the complexity of mechanisms in charge of maintaining equilibrium of Fe in the body. The iron is vital to the life of cells, but its presence in excess is rather toxic. Iron is mostly required for hemoglobin synthesis. It is recycled between reticulo-endothelial macrophages and bone marrow that is the main user of iron. Intestinal absorption is a key step in determining iron capital in the body. Its rate is tightly controlled by several factors that act under influence of signals of unknown nature, which indicate iron needs and storage. The IRP/IRE (iron regulatory protein/iron responsive element) system controls cellular uptake, stores and exportation of iron, and heme synthesis. Mitochondrion is a dynamo of iron metabolism, being vital for heme synthesis and iron sulphur cluster genesis. The recent discovery of several mitochondrial proteins involved in iron metabolism resulted in better understanding mitochondrial iron movement, storage and exchange. Nevertheless, much remains to be known on the role of some actors such as HFE protein, hepcidin, hemojuvelin and transferrin receptor 2, and to determine the nature and mechanisms of signals regulating iron level in the body.
本文是对铁代谢知识的最新综述,整合了该领域的最新研究成果。在理解蛋白质因子(转运蛋白、酶和调节因子)在铁代谢中的作用以及相关遗传异常方面取得了重大进展。该研究突出了负责维持体内铁平衡的机制的复杂性。铁对细胞生命至关重要,但其过量存在具有毒性。铁主要用于血红蛋白合成。它在网状内皮巨噬细胞和骨髓(铁的主要使用者)之间循环。肠道吸收是决定体内铁储备的关键步骤。其速率受到多种因素的严格控制,这些因素在未知性质信号的影响下起作用,这些信号指示铁的需求和储存情况。IRP/IRE(铁调节蛋白/铁反应元件)系统控制细胞对铁的摄取、储存和输出以及血红素合成。线粒体是铁代谢的动力源,对血红素合成和铁硫簇的产生至关重要。最近发现了几种参与铁代谢的线粒体蛋白,这有助于更好地理解线粒体铁的移动、储存和交换。然而,对于一些参与者如HFE蛋白、铁调素、血色素沉着症蛋白和转铁蛋白受体2的作用,以及确定调节体内铁水平的信号的性质和机制,仍有许多有待了解之处。